AB0748 Anti-Fibrotic Therapy in Progressive Pulmonary Fibrosis Associated with Sytemic Sclerosis: Characteristics of SSc-İAH Patients Receiving Nintedanib and Advers Events during Treatment
Authors: Y. Yalçinkaya, S. Amikishiyev, N. Aliyeva, B. Artim-Esen, A. Gul, Z. Bingol, G. Okumuş, M. Inanc
Journal: Annals of the Rheumatic Diseases (2022)

Background Nintedanib, an intracellular inhibitor of tyrosine kinases, has been recently approved for interstitial lung disease associated with systemic sclerosis (SSc-ILD). Nintedanib has shown antifibrotic and antiinflammtory effects in animal models of fibrosing ILDs. Objectives We aimed to evaluate clinical charcteristics of progressive SSc-ILD patients receiving anti-fibrotic therapy who were resistant to standart immunosuppressives (ISs) and adverse events during treatment period in this cohort. Methods Fifteen patients fulfilling ACR/EULAR (2013) classification criteria for SSc and receiving nintedanib for progressive ILD despite standart ISs included into this retrespective analysis. Results Demographics and clinical characteristics of SSc patients were summarised in Table 1. Median age, duration of Raynaud’s and duration of non-Raynaud symptom were 49 (35-72), 8 (1-30) and 4 years (1-21), respectively. ILD was evident median 2 years (0.5-20) after onset of Raynaud’s and 1 years (0.5-11) after onset of non-Raynaud symptom. Before pulmonary involvement, 7 patients received methotrexate and 1 patient cyclophosphamide (CYC) for diffuse cutaneous involvement. After evident ILD, 6 patients received CYC, 5 patients mycophenolate mofetil (MMF) and 4 patients azathioprine as first ISs for SSc-ILD. Median FVC(%) and DLCO(%) were 56 (39-67) and 44 (20-67) before antifibrotic therapy. Thirteen patients (%92,8) received ≥2 ISs before nintedanib. Duration of evident ILD to onset of antifibrotic therapy was median 5.5 years (2-11). Nintedanib was prescribed concomitantly with MMF in 8 or rituksimab-MMF in 6 patients. Median follow-up of antifibrotic treatment period was 7 months (2-18). Advers events during nintedanib were summarised in Table 2. One patient was deceased due to small cell lung cancer at 9.month of nintedanib. Table 1. Characteristics of SSc-ILD Patients Receiving Nintedanib n (%) Females 12 (80) Clinical Characteristics Diffuse Cutaneous SSc 13 (86.7) Limited Cutaneous SSc 2 (13.3) Synovitis 3 (20) Digital ulsers 8 (53.3) Pulmonary arterial pressure &gt;30 mmHg (echo) 5 (30) Gastrointestinal involvement 10 (76.9) Serology ANA 13(86.7) Anti-Scl70 10 (66.7) Immunosuppressives CYC 8 (53.3) MMF 14 (93.3) AZA 7 (46.7) MTX 6 (40) RTX 6 (40) Low dose steroids 15 (100) Table 2. Advers Events during Nintedanib Treatment in SSc-ILD patients SSc-ILD (n=15 ) Any advers events n(%) 8 (53.3) Diarrhoea 5 (30) Nausea/ vomiting 1 (6.7) Abdominal pain 3 (20) Weight decreased 3 (20) Yorgunluk 2 (13.3) Cough 3 (20) Infections (pneumonia) 3 (20) Liver test abnormalities 4 (26.7) Malignancy 1 (7.1) Dose reduction and reescalation 7 (46.7) Dose interruption 2 (13.3) Conclusion Nintedanib was prescribed in progressive SSc-ILD patients who had predominatly diffuse cutaneous involvement, anti-SCL70 positivity and exposed to multiple standart ISs. Duration of ISs were higher than 5 years before antifibrotic therapy. Gastrointestinal (GI) advers events were frequent during nintedanib requiring dose reduction in half of the patients. Nintetanib can be used in progressive SSc-ILD patients considering some difficulties in such a disease with GI involvement. Efficacy analysis of the antifibrotic therapy needs further studies including long term follow-up. Disclosure of Interests None declared